Brachydactyly Cosmetic Surgery

• Avoid distraction osteogenesis at the phalangeal level in brachydactyly.

Diagnostic Studies

Radiographs of the chest and shoulder were normal. Several views of the hands revealed mild webbing of the second and third web spaces and shortened digits on the right hand, primarily at the middle phalangeal level (Fig. 78-2).

Differential Diagnosis

Brachydactyly associated with Poland's syndrome Clinodactyly

Figure 78—1. A 5-year-old child with small right hand and mild syndactyly of the second and third web spaces.

Figure 78—2. (A) Oblique radiographs of both hands with shortening of the right hand, primarily of the middle phalanges. Mild webbing of the soft tissues in the second and third web spaces is apparent. (B) Anteroposterior radiographs of both hands showing the shortened digits ofthe right hand when compared with the left.

Figure 78—2. (A) Oblique radiographs of both hands with shortening of the right hand, primarily of the middle phalanges. Mild webbing of the soft tissues in the second and third web spaces is apparent. (B) Anteroposterior radiographs of both hands showing the shortened digits ofthe right hand when compared with the left.

Camptodactyly

Pseudohypoparathyroidism

Pseudopseudohypoparathyroidism

Clinodactyly is angular deformity of the digit in the coronal plane, usually of the small finger in a radial direction. The middle phalanx is usually asymmetrically short with an inclination toward the ring finger. Camptodactyly is a nontraumatic proximal interphalangeal joint contracture with angulation in the sagittal plane. Pseudohypoparathyroidism or pseudopseudohypoparathyroidism is an X-linked or autosomal-dominant condition associated with brachydactyly of the ring and small digits at the metacarpal level. The shortened metacarpals produce an appearance of absent knuckles of these digits upon forming a fist (knuckle, knuckle, bump, bump). The child is of short stature and often obese.

Diagnosis

Brachydactyly

Brachydactyly is used as a generalized term for digital hypoplasia. However, brachy-dactyly strictly refers to a short hypoplastic digit that has the normal complement of bones. The shortening usually involves the middle phalanx, which is the last phalanx to ossify. The index and small fingers are most commonly affected. The size and length discrepancy of the digit do not improve with growth and may increase throughout childhood. The extent of shortening is variable, and it influences the treatment decision process.

Brachydactyly can also occur in conjunction with numerous syndromes, especially Poland's syndrome. Poland's syndrome is usually sporadic in occurrence and combines hand hypoplasia with chest wall abnormalities. The most common hand anomaly is symbrachydactyly (syndactyly combined with brachydactyly), and the typical chest abnormality is ipsilateral absence of the sternocostal portion of the pectoralis major muscle (Fig. 78—3). However, more severe hand and chest wall

Figure 78—3. A 7-year-old boy with Poland's syndrome and absence of the sternocostal portion of the pectoralis major muscle. The clavicular portion is present.

problems can occur, such as absence of digits, breast, and nipple. Brachydactyly can also occur with other syndromes, including de Lange's, Treacher Collins, and trisomy 21.

Nonsurgical Management

The most important initial intervention is to provide the family with accurate information and resources to help the parents emotionally cope with their child's disability. The parents need to be educated about this congenital anomaly and their emotional distress addressed during the initial visits. We explain to the parents that the brachydactylous hand will always be smaller than the noninvolved side and the discrepancy may increase slightly during growth. If the child has Poland's syndrome, the chest wall abnormalities are also discussed. The asymmetry of the chest is more of a cosmetic than functional deficit and is especially problematic in females.

The treatment of brachydactyly varies with the extent of involvement and status of the other digits. Mild digital shortening requires no treatment, as it has little effect on hand function. The child is able to grasp, pinch, and release without difficulty with the shortened digits. Adaptive equipment can be used to supplement hand function for a particular task that cannot be accomplished (e.g., tying shoes, playing musical instruments).

Figure 78—4. Preoperative examination of the incomplete syndactyly between the shortened index-long and long-ring digits.

Surgical Management

Mild brachydactyly does not interfere with function and does not require surgery. Attempts at improving the appearance of the shortened digit should be avoided for fear of disrupting function. Moderate brachydactyly isolated to a single digit usually does not require treatment, as there is little handicap.

Brachydactyly combined with syndactyly is often associated with Poland's syndrome. The syndactyly is often simple and incomplete and requires separation of the digits with web space reconstruction beginning at about 1 year of age (Fig. 78—4). The commissure should be placed more proximal than normal to compensate for the shortened digits (Fig. 78—5). We prefer a dorsal flap for commissure reconstruction, as this skin is more pliable and re-creates the normal slope of the web space. Syndactyly separation achieves independent digital function, enhances the functional capacity of the hand, and improves appearance. However, the underlying skeletal deficiencies limit the overall performance of the hand.

Digital lengthening is another alternative in brachydactyly and can be accomplished by a variety of techniques. The finger can be lengthened by using an intercalary bone graft or distraction osteogenesis. Digital lengthening by distraction allows the development of longer digits with normal sensation that can enhance function. The site of lengthening varies with the deficient segment. These techniques are used more commonly in brachydactyly from a shortened metacarpal or in hypoplastic digits without the normal complement of phalanges. Brachydactyly

Figure 78—5. Web space reconstruction between the index and long digits using a dorsal flap for commissure reconstruction and a full-thickness skin graft from the

with shortening of the middle phalanx is extremely difficult to lengthen and the complicate rate is high.

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